Zielen S, Sehrt P, Rahmig J, Hofmann D, Brière F, Meuer S C
Abteilung Allgemeine Pädiatrie II, Universität Frankfurt/Main.
Immun Infekt. 1994 Feb;22(1):24-5.
Common variable immunodeficiency (CVI) is widely believed to involve a primary B cell defect, however, there is increasing evidence of T cell dysfunction in these patients. To test the hypothesis that B cells of patients with CVI are not defective, we investigated whether immunoglobulin production can be induced by activation through the CD40 system. Our results show that 6 of 7 children with CVI produced IgG when B cells were activated through CD40 plus IL-10. In most patients, IgM and IgA production were also induced. However, we could demonstrate that failure of immunoglobulin production was not due to a defective CD40 ligand expression on T cells. Taken together, our data show that B cells of patients with CVI are functionally normal when activated through CD40 plus IL-10. IL-10 seems to be more effective than IL-2 in restoration of immunoglobulin production in such patients.
普通可变免疫缺陷(CVI)被广泛认为涉及原发性B细胞缺陷,然而,越来越多的证据表明这些患者存在T细胞功能障碍。为了验证CVI患者的B细胞无缺陷这一假设,我们研究了通过CD40系统激活是否能诱导免疫球蛋白产生。我们的结果显示,7名CVI儿童中有6名在B细胞通过CD40加IL-10激活时产生了IgG。在大多数患者中,也诱导产生了IgM和IgA。然而,我们能够证明免疫球蛋白产生失败并非由于T细胞上CD40配体表达缺陷。综上所述,我们的数据表明,CVI患者的B细胞在通过CD40加IL-10激活时功能正常。在恢复此类患者的免疫球蛋白产生方面,IL-10似乎比IL-2更有效。